A Woman's Journey With Rare Disease

New Year, New Lungs?

I had my latest transplant team appointment yesterday. I am always nervous as hell before them because I can never anticipate what’s really going to happen. I also dread the pulmonary function testing (PFT) because it has become a necessary evil more often than I’d like (which I understand), but it takes a bit more out of me each time.

In the past, I used to religiously track my lung function numbers down to the very statistic & percentage. But I found over time that it made those hits to my lungs harder for me to take.

I would be incredibly angry a few days after because I was reacting to the decline. Resentful of LAM having the upper hand & stealing function away from my hard-working warrior lungs who were doing their best to fight back against the onslaught of the disease. (I used to have 10 point or more drops in a single year. These weren’t just little spikes of a few points here or there.)

Once I started my drug treatment in 2009, I felt better; had more energy. The function testing was spaced further apart so I felt like I could really live outside those numbers. I didn’t even actively solicit the numbers after I had the testing. I figured if there was a concern, my pulmonologist would tell me. It went that way for awhile, even with my transplant team check-ins when they started in 2010/2011 (after my first talk about facts & figures). I had to go back to tracking this April when things definitely changed again, yet I register the numbers & then put them out of sight, out of mind, outside the doctor’s office. I process things better that way.

Yet, Monday night it finally dawned on me how sick I’ve become & how I feel it hit me over the past few months. It’s a hard thing for me to admit. I’m always adapting, compensating, finding a way through. Telling myself my life is not that bad. My quality of life is dying off, albeit by increments & inches. I still have a decent amount left, but even that decent amount takes more work to maintain month-to-month.

I knew this was all a signal that I am physically ready for transplant. That I had long passed the corner of being “too healthy” for this option to be off of the table.

But… was I emotionally or mentally ready? I knew I was close, yet was still feeling extremely unsettled for a reason I couldn’t quite put my finger on Monday night. Now, I know it’s natural to be scared. Probably abnormal if I wasn’t at least a little. These decisions aren’t to be made lightly.

The last appointment we had all talked about listing if I showed more decline. I’d also get an estimate of a wait time frame. They were going to pull me off the drug. I had no clue what to expect on any of this.

(I had given them a full update on the car accident after I returned home in late October, so I wouldn’t waste appointment time having to break that all down for them. They could just ask any relevant quick questions they wanted to, if they wanted to. I was sincerely hoping though that jerk that recklessly plowed into me wasn’t going to interfere with any of this in the grand scheme of things. In my heart-of-hearts I didn’t think so, but I couldn’t really know until we were all in the room.)

I had my PFT before meeting with my doctor & nurse practitioner. Another 4 points down that I’ll never get back with these lungs. I’m not even going into actual percentage that my original lungs have left anymore because I don’t like seeing it. It’s past the point of really getting hung up on too much. It’s definitely at least 8 points under my numbers in April now. (Let’s say it’s definitely less than 30 percent left).

In the end, we all agreed the decline was probably not the result of the car accident in October. I was having drops & changes before it happened. Yet, since it’s only been about a month, we all still felt doing a repeat in December to get additional confirmation was in order first before making any further decisions on listing.

I asked my questions that I had about inactive versus active status & listing. I knew I couldn’t get an “official” lung allocation score right now with the limited data they had & without them actually activating me & putting me on the list. Yet, I asked for a range as far as my overall status now was because I needed to have a ballpark for myself.

Was I at the bottom, barely listable? Or what? The answer I received was surprising. I’m mid-range scoring wise. So I definitely warrant & need this, but I was actually higher in priority than I had assumed I was. This was both good & bad news for me to mull over. The “bad” is only having affirmation that my truly feeling sick is now the hard reality of the situation (in my medical record as well).

What do you anticipate (roughly) my wait time will be? That answer was also a bit better news than I expected.

Based on my blood type & available donors, I may be transplanted sooner than I anticipated. Probably around 6 months, maybe even less once I’m listed. I had prepared myself for the news that it might be a year. That seemed to me to be a relief.

So now the updated plan is most likely listing sometime early next year. Definitely after the holidays.

That makes me feel better too because then I can get out when I feel like it, live a bit more, & get some distance between hospital stays. I agree with my team on that score. Car accident aside, I don’t think I would have felt any differently in the end at the decision we all arrived at. (My gut was just saying it’s not the time now).

Meanwhile, I’ll stay on the drug for at least another month since it’s still working for my kidneys & we need to give them as much of a head start as possible.

There are instances in both TSC & LAM where people with both diseases potentially need or have to have 2 transplants inevitably because of complications. The immunosuppressant drugs can be hard on the kidneys.

However, this is not an absolute so I don’t think about it. Just acknowledge it as a real reality for some people which I feel is important to do. I just feel better since the drug is working on my kidneys yet to keep going with it (even if its not holding my lungs anymore) to avoid having to take the hit from TSC for as long as possible. Let me just duke it out with LAM for now & without the one-two punch of TSC added to the mix.

Now, I feel completely settled because we all are on the same page (as we usually are in each appointment when we’re talking with each other). I’m truly lucky to have such a fantastic team who knows me & my diseases so well.

I’ll be emotionally ready when we all decide to finally pull the trigger on listing, whenever that happens.